Immunotherapy for Neuroendocrine Neoplasms

Cancer immunotherapy has completely changed our views on how cancer can and should be treated. However, although immunotherapy represents a breakthrough in the management of cancer, so far only a minority of cancer patients truly benefits from the treatment and many cancer types are not responding to immunotherapy. For neuroendocrine neoplasms the immunotherapy breakthrough has not yet occurred. A major reason appears to be that the immune landscape of neuroendocrine neoplasms is cold with relatively few effector immune cells infiltrating the tumors. A second reason is that the mutational burden in neuroendocrine neoplasms is relatively low, meaning that immunogenic epitopes for T cells to recognize are sparse. In this chapter, we provide an overview on the current knowledge about immunotherapy with immune checkpoint inhibitors, oncolytic viruses, cancer vaccines, and chimeric antigen receptor (CAR)-T cells.